Outpatient Pediatric Cardiology: Evaluation and Management of Common Conditions - LHPNC 2019

UAE 5th Latifa International Pediatrics &
Neonatology Conference

Outpatient Pediatric Cardiology:
Evaluation and Management of
Common Conditions
Paul E. Farrell, Jr, MD, MBA
Pediatric Cardiologist
The Children’s Hospital of Philadelphia
Clinical Professor of Pediatrics
University of Pennsylvania School of Medicine

March 30, 2019

Objectives

– Review clinical evaluation of cardiac conditions
   • Vital signs, Pulse oximetry, BP (upper and lower
     extremities), Murmurs (Innocent versus
     pathologic), Pulses
– Brief comment on palpitations
– Review the evaluation and management of chest
  pain
   • functional, non-cardiac, or cardiac
– Review the evaluation and management of
  Syncope
   • Vasovagal (neuro-cardiogenic)
   • Cardiac- structural or arrhythmia induced

Cardiac Clinical Evaluation
• History
  – Infants
     • Prenatal history: Maternal diabetes, medication use,
       nuchal thickening, amniocentesis results, other organ
       system abnormalities, prematurity
     • History of respiratory distress, poor growth, feeding
       problems, or cyanosis
  – Children and adolescents
     • Frequent respiratory problems, exercise intolerance,
       chest pain, dizziness, syncope
  – Family history, social history

Cardiac Clinical Evaluation
• Physical exam
   – Growth: height, weight, HC in infants
   – Vital signs: HR, RR and work of breathing, BP (sitting with
     feet on the floor), 4 extremity BP in infants
   – Pulse oximetry:
       • Cyanosis not seen until saturation

Cardiac Clinical Evaluation
• Palpitation
   – Pectus abnormalities, active precordium, thrills
• Auscultation
   – Heart sounds (S1, S2, S3, S4)
      • Narrow or single loud S2- pulmonary hypertension
      • Widely split S2- ASD, RBBB, PAPVC
   – Clicks
      • Ejection clicks in early systole, pulmonary clicks at ULSB,
         aortic clicks at LLSB
      • Mid-late systolic clicks-MVP
      • PDA and Ebstein anomaly- multiple clicks
   – Rubs- seen in pericarditis, sitting, LLSB or apex

Characteristics of Murmurs
– Age at appearance
   • Outflow tract murmurs can be heard in the first hours
     of life
   • As PVR drops in in newborns, shunt murmurs (PDA,
     VSD, AVC) can be heard
– Timing during cardiac cycle
   • Systolic
       – Ejection-crescendo/decrescendo, AS or PS
       – Holosystolic- VSD, mitral valve regurgitation
   • Diastolic- pathologic, AI/PI, flow rumbles in ASD/VSD
   • Continuous- PDA, AV malformations, coronary fistula
   • To-and Fro- TOF with absent pulmonary valve

Characteristics of Murmurs
– Location, radiation, postural maneuvers
    • Note where murmur is loudest on chest
    • Standing increases the murmur of hypertrophic cardiomyopathy
      with outflow tract obstruction (decreases venous return to smaller
      LV, increasing gradient)
    • Squatting increases venous return and LV volume, moves click and
      murmur to a later part of systole in MVP
– Loudness, pitch, and quality (harsh, blowing, to and fro, musical,
  vibratory)
    • Systolic murmurs Grade 1-6, diastolic murmurs 1-4
    • Grade 4, palpable thrill
    • Murmurs diminished with decreased cardiac output (critical aortic
      stenosis in a newborn)

Innocent murmurs
• Typically soft systolic murmurs < grade 4,
  not associated with heart disease
   – Still’s murmur-loudest in supine position,
     decreases with sitting
   – PPS (newborn)
   – Venous hum-best heard sitting, decreases in
     supine position and with neck vein
     compression

Other important physical findings
• Lung exam- rales, wheezing
• Abdominal exam- hepatosplenomegaly,
  ascites
• Distal pulses- Femoral artery pulses,
  diminished, delayed, or absent
• Extremities- clubbing, increased arm span,
  upper to lower segment ratio (Marfan),
  edema, capillary refill

Palpitations and Irregular rhythms
• Premature atrial contractions
   – Common in fetus and neonates, usually benign and resolve
      over a few months if infrequent
• Premature ventricular contractions
   – Usually require cardiac evaluation
• Evaluation
   – History and physical exam
   – EKG with rhythm strip, Holter monitor, looping event
      monitors
• Treatment depends on type and frequency of events
• Abnormal EKG, rapid/irregular rhythms (SVT, atrial
  flutter, frequent PVC’s)- recommend cardiac consultation

Chest Pain in Children and Adolescents

• Etiology
  –Idiopathic                     40%
  –Musculoskeletal                30%
  –Hyperventilation/Emotional     20%
  –Breast related                 5%
  –Respiratory/Gastrointestinal   4%
  – Cardiac                       1%

Chest Pain in Children and Adolescents

• History most important in evaluation
• Cardiac etiology of chest pain not likely if:
  – Unrelated to exercise
  – Patient has systemic illness
  – Negative cardiac exam and EKG
• Chest pain with exertion and not at rest, or
  worse in supine position requires further
  workup

Chest Pain in Children and Adolescents

• Costochondritis
  – Usually unilateral, 2-3 continuous upper
    costochondral or sternal joints
  – Sharp pain, lasting seconds-minutes
  – Worse with deep breathing
  – Pain reproduced by pushing on joints
  – Treatment- anti-inflammatory medications,
    heating pad, physical therapy, localized
    steroid injections

Chest Pain in Children and Adolescents
• Respiratory Causes:
   – Asthma
   – Infection (pneumonia, bronchitis, pleurodynia)
   – Pneumothorax (abrupt onset, severe pain, SOB)
• Pulmonary vascular tree causes
   – Primary pulmonary hypertension
   – Pulmonary embolism (acute onset, severe SOB, pleuritic
     CP). Order d-dimer, oxygen saturation, CXR, CT
     angiogram
• GI causes
  – Esophageal reflux or spasm, dysmotility, foreign body
  – Ulcer

Chest Pain in Children and Adolescents

• Pericarditis
  – Etiology: idiopathic, infectious, systemic illness
    (SLE), post- pericardiotomy, trauma, uremia,
    neoplastic
  – Symptoms: substernal or left sided chest pain,
    pain worse in supine position and less in sitting
    position
  – Symptoms: Fever (2/3), friction rub (1/4), pulsus
    paradoxus (abnormally large decrease in pulse
    amplitude and systolic BP during inspiration)

Chest Pain in Children and Adolescents

• Pericarditis
  –Evaluation: CBC, Inflammatory
   markers, troponin (associated
   myocarditis), EKG, echocardiogram
  –Treatment: anti-inflammatory
   medications, steroids,
   pericardiocentesis for significant
   effusions with tamponade

Chest Pain in Children and Adolescents

• Cardiac causes:
  – Aortic stenosis
  – Hypertrophic cardiomyopathy
  – Coronary artery anomalies
     • Anomalous origin of the left coronary artery
       from the pulmonary artery (ALCAPA)
     • Anomalous origin of the LCA or RCA from the
       opposite sinus of Valsalva
• Evaluation: exam, EKG, echocardiogram, exercise
  testing, CT or MRI, cardiac catheterization

Etiology of Syncope
• Syncope is caused by an interruption of
  blood flow to the brain
• The single most common reason for syncope
  in children and adolescents is an abrupt
  change in vasomotor tone known as
  vasovagal syncope
• Generally the conditions are grouped into
  cardiac and non-cardiac causes

Vasovagal Syncope
• Exaggeration of these responses leads to a
  drop in blood pressure through several
  mechanisms
 – Hypotension (independent of heart rate) –
   vasodepressor type
 – Sinus bradycardia – cardio-inhibitory type
• Usually specific triggers can be identified
 – Postural changes, hair brushing, coughing, micturition,
   shaving, emotions, pain, swallowing, etc.
• Accompanied by a prodrome / pre-syncopal
  symptoms

Vasovagal syncope and exercise

• The benign exercise related syncope...
  – Must exclude cardiac etiologies carefully
• Commonly occurs after the termination of
  activity
  – Collapses after walking to sideline, not during activity
• Usually has the same prodrome as common
  fainting

Cardiac Causes of Syncope

• Structural                    • Primary arrhythmia
  – Aortic stenosis                 –  LQTS
  – Cardiomyopathy                  –  SQTS
      • HCM, DCM, acute             –  Brugada
  – Coronary artery                 –  CPVT
    anomalies                       –  Idiopathic VF
  – ARVD                            –  WPW with rapid
  – Pulmonary HTN                      ventricular response
  – CHD                              – Bradyarrhythmias
  These are the etiologies that need to be referred due to
     an increased risk of sudden cardiac death (SCD)

Structural
• Aortic stenosis
  – Limited cardiac output due to physical obstruction
  – Sudden death likely related to arrhythmias, not
    obstruction, due to ischemia
• Coronary anomalies
  – Anomalous origins of coronary arteries
     • Particularly left coronary arteries from the right sinus
     • Again, SCD due to arrhythmias secondary to ischemia
  – Muscular bridge

Structural
• Arrhythmogenic right ventricular dysplasia
 – Typically an etiology in older children/adolescents
   • Mean age of presentation is 30 years
 – Fatty infiltration of the RV leading to ventricular
   arrhythmias
• Cardiomyopathy
 – Due to arrhythmias in dilated cardiomyopathy and acute
   myocarditis in the setting of poor output
 – Syncope in hypertrophic cardiomyopathy also likely due to
   ventricular arrhythmias but may also have some element of
   obstruction to outflow contributing to symptoms
   • Hypertrophic cardiomyopathy is the most common cause of
     sudden cardiac death in children and adolescents

Structural
• Pulmonary hypertension
  – Due to any number of etiologies
  – Can have RV arrhythmias if RV function is poor
  – Usually due to acute increases in pulmonary pressures
    with subsequent decrease in cardiac output
• CHD
  – Usually later in life related to poor function plus
    arrhythmias in complex CHD
     • TOF, TGA, AS are particularly at risk for sudden cardiac
       death
  – Some may present due to obstruction

Arrhythmia
• Long QT syndrome
  – Prolongation of the QT interval due to abnormal
    repolarization
  – Predisposes to polymorphic ventricular tachycardia
     • Torsades de pointes
  – Can be inherited or acquired
     • Inherited – types 1, 2 and 3 are most common
     • Acquired – drugs, electrolyte abnormalities (low K, low
       Ca, low Mg), hypothyroidism, bradycardia

Arrhythmia
• Brugada
  – Channelopathy resulting in ventricular tachycardia
  – Usually male dominant, SE Asian, and older
    population (older adolescents)
  – More common during fever to have arrhythmia
• Catecholaminergic polymorphic ventricular
  tachycardia (CPVT)
  – Channelopathy resulting in polymorphic ventricular
    tachycardia
  – Related to catecholamine stimulation
     • Emotion, exertion

Arrhythmia
• Wolff-Parkinson-White syndrome with rapid
  ventricular response (WPW with RVR)
  – An accessory pathway provides an alternative route
    for atrial signals to reach the ventricle
  – If the atrial rhythm is very fast it can conduct rapidly
    and lead to ventricular fibrillation
  – Not all accessory pathways can do this
• Short QT syndrome
  – Channelopathy leading to abnormal repolarization
    predisposing to ventricular arrhythmias
  – Very rare

History
• HPI
  – What they were doing when they passed out?
     • Exertional syncope is ALWAYS a cause for concern
     • Syncope AFTER exertion is usually vasovagal but still
       needs to be investigated
  – Where there any prodromal symptoms?
  – How long were they out?
  – Any symptoms while unconscious?
     • Was anyone around to witness the event?
  – How often does it occur?
  – Is it always in the same circumstances?

History
• PMH
  – Any significant cardiac, psychiatric or neurologic
    history
• FH
  – This is absolutely key – BE VERY SPECIFIC
  – History of sudden unexplained deaths, especially in
    those younger than 55 years
       • Unexplained drowning, car accidents, SIDS
  – History of syncope
  – History of arrhythmia disorders (e.g., pacer or ICD?)
  – History of cardiomyopathy

Physical Exam
• Make sure orthostatics are part of the vital
  signs
  – Laying, sitting, standing, standing 3 minutes
  – HR increase > 30 BPM- Postural Orthostatic
    Tachycardia Syndrome (POTS)
• A thorough neurologic exam can be useful to
  document focal lesions
• A good cardiac examination to evaluate for
  structural disease
  – Most cardiac etiologies will not be picked up this way

Case 1
• 15 y/o female presents to primary care clinic for
  recurrent syncope
• Occurs most often after standing long periods of time
  and when she is having blood tests
• Collapses, convulses, awakens
• PE normal
• ECG normal
• FH benign
• Diagnosis: Vasovagal (also known as neuro-
  cardiogenic syncope)

Case 2
• 13 y/o previously healthy girl with no history of
  syncope or pre-syncope collapses while
  playing soccer
  –   Stays unresponsive
  –   Bystanders “feel” a pulse
  –   AED applied and shocks patient
  –   Patient wakes up, but feels bad
  –   Reports no prodrome prior to the collapse
  –   Never had more extensive workup than a sports physical
  –   Sibling died of SIDS (unknown to patient)
• Historical red flags?

Case 2 – Long QT syndrome

RR = 600 ms

    QT = 400 ms

                  QTc = QT/√RR = 0.4/√0.6 = 515 ms

Case 3
• 16 y/o previously healthy boy collapses while
  playing basketball
• No prodrome, he does not recall falling
• Collapses two more times during time out
• Positive FH of sudden early cardiac death
• Exam in hospital unremarkable
• EKG and echocardiogram performed

Case 3 – Hypertrophic Cardiomyopathy

References
•   Thompson, W.R., Reinisch, A.J., Unterberger, M.J. et al. Pediatr Cardiol
    (2018). Artificial Intelligence-Assisted Auscultation of Heart Murmurs:
    Validation by Virtual Clinical Trial. Sensitivity and specificity for detection
    of pathologic cases were 93% (CI 90–95%) and 81% (CI 75–85%),
    respectively, with accuracy 88% (CI 85–91%).
•   Lu et al, Congenital Heart Disease, November/December Development of
    quality metrics for ambulatory pediatric cardiology: Chest pain. The 3
    approved quality metrics included: (1) documentation of family history of
    cardiomyopathy, early coronary artery disease or sudden death, (2)
    performance of electrocardiogram in all patients, and (3) performance of an
    echocardiogram to evaluate coronary arteries in patients with exertional
    chest pain
•   Stewart, et al Pediatrics, January 2018, Vol 141 Pediatric Disorders of
    Orthostatic Intolerance (review article)